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A paraganglioma is a rare neuroendocrine neoplasm that may develop at various body sites ... By histochemistry, the paraganglioma cells are argyrophilic, ...
A paraganglion (pl. paraganglia) is a group of non-neuronal cells derived of the neural crest. They are named for being generally in close proximity to sympathetic ganglia. They are essentially of two types: (1) chromaffin or sympathetic paraganglia made of chromaffin cells and (2) nonchromaffin or parasympathetic paraganglia made of glomus cells.
A gangliocytic paraganglioma is a rare tumour that is typically found in the duodenum and consists of three components: (1) ganglion cells, (2) epithelioid cells (paraganglioma-like) and, (3) spindle cells (schwannoma-like). [1]
Pheochromocytoma is a rare tumor of the adrenal medulla composed of chromaffin cells and is part of the paraganglioma (PGL) family of tumors, being defined as an intra-adrenal PGL .
A zellballen is a small nest of chromaffin cells or chief cells with pale eosinophilic staining. Zellballen are separated into groups by segmenting bands of fibrovascular stroma, and are surrounded by supporting sustentacular cells. [1] A zellballen pattern is diagnostic for paraganglioma or pheochromocytoma. [2] Zellballen is German for "ball ...
[4] [5] When a distinction is made, the "aortic bodies" are chemoreceptors which regulate circulation, while the "paraaortic bodies" are the chromaffin cells which manufacture catecholamines. Organs of Zuckerkandl (O of Z) harbor the potential for deadly paragangliomas. Paragangliomas are one of the surgical causes of hypertension.
Glomus tumors are modified smooth muscle cells that control the thermoregulatory function of dermal glomus bodies. As stated above, these lesions should not be confused with paragangliomas, which were formerly also called glomus tumors in now-antiquated clinical usage. Glomus tumors do not arise from glomus cells, but paragangliomas do.
Germline mutations in the gene can cause familial paraganglioma (in old nomenclature, Paraganglioma Type PGL4). The same condition is often called familial pheochromocytoma. Less frequently, renal cell carcinoma can be caused by this mutation. Paragangliomas related to SDHB mutations have a high rate of malignancy.